Sickle Cell Disease Flashcards
What is the typical lifespan of a RBC?
- ~120 days
What is sickle cell disease?
- group of inherited RBC disorders (abnormal hemoglobin - Hemoglobin S that gives it the “sickle shape”) that common affects the African american population
What is the average RBC lifespan with sickle cell?
- 10 - 20 days (causing the anemia)
what is vaso-occlusive crisis?
- When oxygen is prevented from reaching the tissus = pain (can occur in the extremities)
What is important to know about the infection risk associated with sickle cell disease
- SCD causes functional Aspleenia - causing increased risk of serious infections; may need immunizationa nd prophylactic antibiotics
Strep Pnumo, H. Flu, N. Meningitidis are the most common bugs
What are some non drug treatments for sickle cell disease
- Blood Transfusions (Hgb goal no higher than 10)
- Can do Chelation therapy to remove excess iron
What are some immunizations and antibiotics someone with sickle cell disease should take/get?
- Vaccination: Hib, Pneumococcal, Meningococcal (recommended)
- Antibiotics: Oral Penicillin BID until age 5 yo (prophylaxis toward S. Pneumo, H. Flu, N. Meningitidis)
What is the purpose for iron chelation treatment and are some of the agents that are used?
- Iron overload from blood transfusion = removes excess iron
- Oral Agents: Deferasirox & Deferiprone
What are some treatment options for someone with sickle cell disease?
- Hydroxurea
- L-Glutamine
- Voxelotor
- Crizanlizumab
What is the MOA for Hydroxyurea
- Stimulate the production of Hgb-F and reduce the pain crises (long term use)
What is the boxed warning for hydroxyurea?
- Myleosuppression
What are some warnings for hydroxyurea?
- fetal toxcitiy
- Avoid live vaccines
What are some additional notes for Hydroxyurea?
- Contracption is REQUIRED
- Hazardous drug = wear goves and wash hands
- Folic acid supplementation is needed
